Journal of Pediatric Surgery (2006) 41, E1 – E4
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Management of children with pancreatic head mass Jinyoung Parka, James C.Y. Dunnb, James B. Atkinsonb,* a
Department of Pediatric Surgery, School of Medicine, Kyungpook National University, Taegu, Korea Division of Pediatric Surgery, David Geffen School of Medicine, University of California, Los Angeles, USA
b
Index words: Obstructive jaundice; Children; Pancreatic head mass; Pancreatitis
Abstract The management of children with a mass in the head of the pancreas is not well defined. The medical records of 3 children with obstructive jaundice because of a mass in the head of the pancreas over a 4-year period were reviewed retrospectively. Abdominal ultrasonography and computed tomography showed intrahepatic and extrahepatic ductal dilatation and a pancreatic mass. Intraoperative frozen section revealed no evidence of malignancy. These patients were separately managed by pyloruspreserving pancreaticoduodenectomy, cholecystectomy and Roux-en-Y choledochojejunostomy, common duct exploration, and T-tube drainage. The final pathology of the pancreatic head mass in all cases demonstrated chronic pancreatitis. Follow-up at an average of 7 months postoperatively showed no recurrence of obstructive jaundice. Unlike adults with a mass in the head of the pancreas, it is recommended that children with similar presentation should undergo biopsy and biliary diversion rather than resection as the primary therapy. Adults presenting with similar radiologic and clinical features would be treated by a pancreaticoduodenectomy in the absence of histologic evidence of malignancy. This series would suggest histologic conformation should be obtained before radical surgery in children. D 2006 Elsevier Inc. All rights reserved.
Obstructive jaundice in childhood encompasses a differential diagnosis that includes choledocholithiasis, pancreatitis, pancreatic pseudocyst, choledochal cyst, intrahepatic neoplasm, and pancreatic tumors [1]. Unlike adults, children rarely develop malignancy of the pancreas. Despite currently available diagnostic imaging modalities, it is very difficult to distinguish a benign pancreatic mass from pancreatic cancer preoperatively. The optimal management of children with a mass in the head of the pancreas is unknown. We reviewed the medical records of 3 children with biliary stricture because of a mass in the pancreatic head. A review of the literature is included for comparison.
Presented at the 16th Annual Conference of the Southern California Chapter of the American College of Surgeons, Santa Barbara, Calif, January, 2005. * Corresponding author. Tel.: +1 310 206 2429; fax: +1 310 296 1120. E-mail address:
[email protected] (J.B. Atkinson). 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2006.02.029
1. Case reports 1.1. Case 1 A previously healthy boy presented at 5 1/2 years of age with a 2-week history of painless jaundice. He complained of abdominal pain and had clay-colored stool, dark urine, easy bruising, and itching. On physical examination, he was icteric, and his abdomen was soft, without a palpable mass. Initial laboratory studies were notable for a total bilirubin of 7.2 mg/dL, conjugated bilirubin of 4.1 mg/dL, alkaline phosphatase of 1298 U/L, serum glutamic-oxaloacetic transaminase (SGOT) of 281 U/L, serum glutamic-pyruvic transaminase (SGPT) of 332 U/L, serum amylase of 109 U/L, serum lipase of 34 U/L, and a negative hepatitis panel. Abdominal ultrasonography (US) and computed tomography (CT) scan showed intrahepatic and extrahepatic ductal dilatation and mild pancreatic ductal dilatation. Endoscopic retrograde cholangiopancreatography (ERCP)
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J. Park et al. measuring 4 5 cm, and the tail and body of the pancreas were inflamed and thickened. The pancreatic mass was obstructing both the bile duct and the pancreatic duct, causing secondary pancreatitis. A pancreaticoduodenectomy was performed. The pathologic diagnosis was chronic pancreatitis. The postoperative course was notable for an episode of intestinal obstruction that required adhesiolysis at 1 year. The decision to proceed with radical resection in this case was based upon the need to relieve obstruction of both the pancreatic and bile ducts, as well as a remote possibility of malignancy.
1.3. Case 3
Fig. 1 Endoscopic retrograde cholangiopancreatography showed the stricture of common bile duct distal to takeoff of the cystic duct.
showed a distal stricture of the common bile duct (Fig. 1). The biliary stricture was fibrotic and did not respond to sphincterotomy. A biliary stent was placed to relieve the obstruction. At operative exploration, he was found to have a firm inflammatory mass in the head of the pancreas, which was not seen on the preoperative radiologic studies. A transduodenal biopsy of the head of the pancreas was performed. Intraoperative frozen section showed pancreatic tissue with benign fibrosis. A cholecystectomy and Roux-en-Y choledochojejunostomy was performed. The postoperative course was uneventful. Follow-up T-tube cholangiogram at 2 months was unremarkable. Five months after operation, a follow-up US showed air in the biliary tract without biliary dilatation.
1.2. Case 2 A previously healthy adolescent boy presented at 15 years of age with a 4-week history of jaundice. He complained of abdominal pain and had clay-colored stool. On physical examination, he was icteric but was otherwise normalappearing. His abdomen was soft, without palpable mass. Initial laboratory studies included a total bilirubin of 5.9 mg/dL, conjugated bilirubin of 3.4 mg/dL, alkaline phosphatase of 400 U/L, SGOT of 105 U/L, SGPT of 380 U/L, amylase of 100 U/L, lipase of 109 U/L, and a negative hepatitis panel. Abdominal US and CT scan showed intrahepatic and extrahepatic ductal dilatation and a pancreatic head mass measuring 3 4 cm. Endoscopic retrograde cholangiopancreatography with brushing cytology was negative for malignant cells. A preoperative fineneedle aspiration biopsy showed normal pancreas with no evidence of malignancy. At operative exploration, the boy was found to have a firm mass in the head of the pancreas
A previously healthy male infant presented at with a 2-week history of jaundice. He was transferred to our hospital after an exploratory laparotomy with pancreatic biopsy performed at another institution. This biopsy was reviewed and showed benign inflammatory pancreatic tissue. On physical examination, he was icteric but was otherwise healthy-appearing. Initial laboratory studies included a total bilirubin of 13.6 mg/dL, conjugated bilirubin of 8.4 mg/dL, alkaline phosphatase of 534 U/L, SGOT of 177 U/L, SGPT of 82 U/L, amylase of 50 U/L, lipase of 19 U/L, and a negative hepatitis panel. Abdominal US and CT scan showed a dilated common hepatic duct and a dilated common bile duct, as well as a pancreatic head mass with enhancement (Fig. 2). Although ERCP showed a normal appearing pancreatic duct, a stent could not be passed through the common bile duct. A percutaneous transhepatic biliary stent was performed to relieve the biliary obstruction. At operative exploration 3 months later, he was found to have a firm inflammatory mass in the head of the pancreas. A transduodenal biopsy of the head of the pancreas was performed. Intraoperative frozen section showed chronic pancreatitis. Intraoperative cholangiogram showed an irregular pattern at the distal bile duct without filling defects. Common duct exploration revealed inflamed common duct mucosa without stones. The stricture was assumed to be inflammatory in nature, possibly
Fig. 2 Abdominal CT scan showed the pancreatic head mass with enhancement.
Management of children with pancreatic head mass related to a history of gallstone pancreatitis. Therefore, no resection was performed. The need for an internal biliary-enteric drainage procedure was considered; however, an external drainage was performed. A T tube was inserted into the choledochotomy site. The postoperative course was uneventful. Follow-up T-tube cholangiogram 2 months after the operation was unremarkable, and the T tube was removed.
2. Discussion Obstructive jaundice in childhood encompasses a differential diagnosis that includes choledocholithiasis, pancreatitis, pancreatic pseudocyst, choledochal cyst, intrahepatic neoplasm, and pancreatic tumors. Pancreatic tumors are uncommon in childhood. They account for 0.2% of all childhood malignancies as compared with 3% in adults [2]. The differential diagnosis of a pancreatic mass in a child is different than that in an adult. Although adenocarcinoma of the pancreas is the most clinically important pancreatic mass in adults, it is rare in children [3]. Pancreatic masses in children may represent an epithelial neoplasm such as adenoma, pancreatoblastoma, solid and papillary neoplasm, tumors of endocrine origin, or nonepithelial tumor, such as lymphoma, primitive neuroectodermal tumor, neuroblastoma, and rhabdomyosarcoma. Chronic pancreatitis with a mass effect shares similar clinical presentations with cancer of the head of the pancreas, such as abdominal and back pain, weight loss, steatorrhea, and obstructive jaundice [4]. Pancreatic cancer can also cause chronic pancreatitis by obstructing the pancreatic duct. It is well known that chronic pancreatitis and pancreatic cancer might coexist. In adults, acute and chronic pancreatitis is usually caused by alcohol abuse or biliary tract disease. In children, the most common etiologies are trauma [5], heredity [6], systemic diseases [7], choledocholithiasis, drug-induced such as didanosine, azathioprine, and mercaptopurine, malformations of the pancreaticobiliary duct system, such as pancreaticobiliary malunion, pancreas divisum, hypertensive sphincter of Oddi and annular pancreas, and idiopathic [8,9]. In our cases, there were no specific causative factors identified. Despite currently available diagnostic imaging modalities, it is very difficult to distinguish preoperatively a benign pancreatic head mass from pancreatic cancer. Computed tomography is the most important imaging modality of the pancreas. Endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography may provide ductal anatomy that is useful for operative planning. The contours of the pancreatic duct are more irregular in chronic pancreatitis than those in tumorrelated obstructions [10-12]. Ragozzino and Scaglione [13] reported that magnetic resonance imaging is much more sensitive and specific
E3 than CT in pancreatic lesion detection. Although not pathognomonic, the typical cholangiographic pattern on magnetic resonance cholangiopancreatography sequences may be helpful in the differential diagnosis between pancreatitis and pancreatic head tumor. Typical cholangiographic patterns secondary to pancreatic cancer are the double duct sign (dilatation of both the biliary and pancreatic ducts) and the mouse tail sign (reduction of the bile duct caliber because of sudden obstruction at the pancreatic head). In chronic pancreatitis, the biliary tract may have a tapered or an irregular appearance on magnetic resonance imaging with alterations of dilated and stenotic biliary segments. Although the pancreatic head masses can be documented by imaging, the correct diagnosis remains difficult. Because the management of patients with stricture of the extrahepatic bile duct depends on the underlying cause, it is useful to obtain tissue for a pathologic diagnosis. Generally, the amount of tissue obtained by ERCP is limited, and the use of brush cytology as an adjunct has proved to be increasingly useful. Cohen et al [14] reported that endoscopic retrograde wire-guided brushing of strictures using 3 cytologic features (nuclear molding, chromatin clumping, and increased unclear-cytoplasmic ratio) to be useful in discriminating malignant from benign stricture of the extrahepatic biliary tract. Even intraoperatively, the differentiation between chronic pancreatitis and pancreatic cancer is difficult. Olah et al [15] reported that intraoperative biopsy had a false-negative rate of 12.5%, and the diagnosis was uncertain in 35%. These data showed that a benign biopsy result does not exclude the presence of malignancy. In cases of mass lesions with obstructive symptoms, resection is a reasonable option in adults, even in the case of chronic pancreatitis. These recommendations in adults are based upon a much higher probability of malignant tumors. The best surgical procedure for children with a pancreatic head mass is much less clear. In adults, chronic pancreatitis with a mass in the pancreatic head is best managed by a pylorus-preserving pancreaticoduodenectomy. Diversion of the biliary tract can also be safely performed using Roux-en-Y hepaticojejunostomy or choledochojejunostomy over a transanastomotic tube. Smith et al [1] reported a child with pancreatic head adenocarcinoma, which was treated with pancreaticoduodenectomy, and suggested that aggressive surgical therapy is not only possible but also essential. In contrast, the present series of cases revealed the benign nature of the masses in the head of the pancreas in all 3 children. Unlike adults with a mass in the head of the pancreas, children with similar presentation should undergo biopsy and biliary diversion rather than resection as the primary therapy, unless histologic evidence of malignancy can be demonstrated. In rare cases, where both the pancreatic and biliary ducts are obstructed, pancreaticoduodenectomy may be indicated even for benign disease.
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